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Transcript Profiles of Stria Vascularis in Models of Waardenburg Syndrome

BACKGROUND: Waardenburg syndrome is an uncommon genetic condition characterized by at least some degree of congenital hearing loss and pigmentation deficiencies. However, the genetic pathway affecting the development of stria vascularis is not fully illustrated. METHODS: The transcript profile of st...

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Autores principales: Chen, Linjun, Wang, Lin, Chen, Lei, Wang, Fangyuan, Ji, Fei, Sun, Wei, Zhao, Hui, Han, Weiju, Yang, Shiming
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Hindawi 2020
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7416267/
https://www.ncbi.nlm.nih.gov/pubmed/32802035
http://dx.doi.org/10.1155/2020/2908182
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author Chen, Linjun
Wang, Lin
Chen, Lei
Wang, Fangyuan
Ji, Fei
Sun, Wei
Zhao, Hui
Han, Weiju
Yang, Shiming
author_facet Chen, Linjun
Wang, Lin
Chen, Lei
Wang, Fangyuan
Ji, Fei
Sun, Wei
Zhao, Hui
Han, Weiju
Yang, Shiming
author_sort Chen, Linjun
collection PubMed
description BACKGROUND: Waardenburg syndrome is an uncommon genetic condition characterized by at least some degree of congenital hearing loss and pigmentation deficiencies. However, the genetic pathway affecting the development of stria vascularis is not fully illustrated. METHODS: The transcript profile of stria vascularis of Waardenburg syndrome was studied using Mitf-M mutant pig and mice models. Therefore, GO analysis was performed to identify the differential gene expression caused by Mitf-M mutation. RESULTS: There were 113 genes in tyrosine metabolism, melanin formation, and ion transportations showed significant changes in pig models and 191 genes in mice models. In addition, there were some spice's specific gene changes in the stria vascularis in the mouse and porcine models. The expression of tight junction-associated genes, including Cadm1, Cldn11, Pcdh1, Pcdh19, and Cdh24 genes, were significantly higher in porcine models compared to mouse models. Vascular-related and ion channel-related genes in the stria vascularis were also shown significantly difference between the two species. The expression of Col2a1, Col3a1, Col11a1, and Col11a2 genes were higher, and the expression of Col8a2, Cd34, and Ncam genes were lower in the porcine models compared to mouse models. CONCLUSIONS: Our data suggests that there is a significant difference on the gene expression and function between these two models.
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spelling pubmed-74162672020-08-14 Transcript Profiles of Stria Vascularis in Models of Waardenburg Syndrome Chen, Linjun Wang, Lin Chen, Lei Wang, Fangyuan Ji, Fei Sun, Wei Zhao, Hui Han, Weiju Yang, Shiming Neural Plast Research Article BACKGROUND: Waardenburg syndrome is an uncommon genetic condition characterized by at least some degree of congenital hearing loss and pigmentation deficiencies. However, the genetic pathway affecting the development of stria vascularis is not fully illustrated. METHODS: The transcript profile of stria vascularis of Waardenburg syndrome was studied using Mitf-M mutant pig and mice models. Therefore, GO analysis was performed to identify the differential gene expression caused by Mitf-M mutation. RESULTS: There were 113 genes in tyrosine metabolism, melanin formation, and ion transportations showed significant changes in pig models and 191 genes in mice models. In addition, there were some spice's specific gene changes in the stria vascularis in the mouse and porcine models. The expression of tight junction-associated genes, including Cadm1, Cldn11, Pcdh1, Pcdh19, and Cdh24 genes, were significantly higher in porcine models compared to mouse models. Vascular-related and ion channel-related genes in the stria vascularis were also shown significantly difference between the two species. The expression of Col2a1, Col3a1, Col11a1, and Col11a2 genes were higher, and the expression of Col8a2, Cd34, and Ncam genes were lower in the porcine models compared to mouse models. CONCLUSIONS: Our data suggests that there is a significant difference on the gene expression and function between these two models. Hindawi 2020-08-01 /pmc/articles/PMC7416267/ /pubmed/32802035 http://dx.doi.org/10.1155/2020/2908182 Text en Copyright © 2020 Linjun Chen et al. http://creativecommons.org/licenses/by/4.0/ This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
spellingShingle Research Article
Chen, Linjun
Wang, Lin
Chen, Lei
Wang, Fangyuan
Ji, Fei
Sun, Wei
Zhao, Hui
Han, Weiju
Yang, Shiming
Transcript Profiles of Stria Vascularis in Models of Waardenburg Syndrome
title Transcript Profiles of Stria Vascularis in Models of Waardenburg Syndrome
title_full Transcript Profiles of Stria Vascularis in Models of Waardenburg Syndrome
title_fullStr Transcript Profiles of Stria Vascularis in Models of Waardenburg Syndrome
title_full_unstemmed Transcript Profiles of Stria Vascularis in Models of Waardenburg Syndrome
title_short Transcript Profiles of Stria Vascularis in Models of Waardenburg Syndrome
title_sort transcript profiles of stria vascularis in models of waardenburg syndrome
topic Research Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7416267/
https://www.ncbi.nlm.nih.gov/pubmed/32802035
http://dx.doi.org/10.1155/2020/2908182
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